Zollinger–Ellison Syndrome

What is Zollinger–Ellison syndrome?

Zollinger–Ellison syndrome is a rare disease that makes the stomach produce far more acid than normal.

In this disease there is usually a tumour called a gastrinoma, which produces the hormone gastrin, in the pancreas or the duodenum.

The hormone gastrin increases the secretion of stomach acid. Producing too much acid can in turn cause recurring ulcers (sores) in the stomach and the duodenum, reflux and diarrhoea.

Important: some of the tumours called gastrinomas can be malignant. This does not mean, however, that the cancer has spread in every patient.

What are the symptoms?

• Ulcers of the stomach and duodenum that keep coming back or are slow to heal
• Pain and burning in the upper abdomen
• Long-lasting or recurring diarrhoea
• Severe stomach reflux
• Nausea and vomiting
• Weight loss in some patients

This disease may be looked for especially when ulcers that keep coming back despite proper treatment are seen together with unexplained diarrhoea.

Why does it happen?

The underlying cause of the disease is a gastrinoma, a tumour that secretes too much of the hormone gastrin.

Most patients have no clear inherited cause. In some people, however, it can be seen together with an inherited condition called MEN1.

How is it diagnosed?

For the diagnosis, the level of the hormone gastrin is first checked with blood tests.

A high gastrin level on its own, however, does not establish a diagnosis of Zollinger–Ellison syndrome. Drugs that reduce stomach acid and other diseases can also raise the gastrin level.

When needed, special tests that assess the level of stomach acid, endoscopic ultrasound (EUS), CT, MRI or special imaging methods can be used.

Is there a treatment?

Yes. The disease can be treated.

The first aim of treatment is to bring the excess stomach acid under control. Strong acid-reducing drugs are usually used for this.

Depending on where the tumour is, how large it is and how far it has spread, surgery or other treatments may also be needed.

Some patients need long-term drug treatment and regular follow-up.

When should you see a doctor?

You are advised to see a gastroenterologist especially if you have stomach ulcers that keep coming back, severe reflux that continues despite treatment, or long-lasting unexplained diarrhoea.

If you vomit blood, pass black stools or have very severe abdominal pain that starts suddenly, you should go to the emergency department.

Remember: not every stomach ulcer or high gastrin level means Zollinger–Ellison syndrome. With the right diagnosis and suitable treatment, stomach acid can be brought under control and the problems caused by the disease can be reduced.